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December 1995

Picture of the Month

Author Affiliations

From the Department of Pediatrics, National Taiwan University Hospital, Taipei (Drs Hou and Wang), and the American Board of Pediatrics, Chapel Hill, NC (Dr Tunnessen).

Arch Pediatr Adolesc Med. 1995;149(12):1391-1392. doi:10.1001/archpedi.1995.02170250097017

AN 8-YEAR-OLD girl was referred for evaluation of short stature (99 cm, less than the third percentile for age). Her birth and medical histories were unremarkable. The family history was negative for short stature or bone disorders. In addition to the features noted in the photographs (Figure 1 and Figure 2), the physical examination revealed a narrow, high-arched palate, delayed eruption of permanent teeth, dental caries, and a nonpalpable right clavicle. Radiographic studies of the chest and skull are shown in Figure 3 and Figure 4.

Denouement and Discussion 

Cleidocranial Dysplasia  Cleidocranial dysplasia is an uncommon generalized skeletal disorder whose name designates the involvement of the cranial vault and clavicles, its most striking features. As more thorough investigation and examination of cases occurred, systemic dysplasia of skeletal and dental tissues became apparent. There is variability in the clinical appearance of affected individuals. The head appears large and round, with frontal,

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